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2014 ; 11
(2
): 311-8
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Autoimmune epilepsies
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Bien CG
; Bauer J
Neurotherapeutics
2014[Apr]; 11
(2
): 311-8
PMID24566940
show ga
In patients with immune-associated disorders of the gray central nervous system
matter (including recurrent seizures), antibodies against intracellular antigens
have been discovered since the 1980s/1990s. In recent years, new antibodies
against surface antigens have also been discovered. In two respects, these
antibodies are even more interesting than the ones to intracellular antigens as,
first, they promise a better response to immunotherapy; and, second, these
antibodies contribute greatly to the understanding of the disease mechanisms.
Whereas in encephalitides with antibodies against intracellular antigens, a
cytotoxic T-cell-mediated response seems to be responsible for neuronal cell
loss, in encephalitides with autoantibodies against surface antigens these
antibodies are probably the relevant pathogenic agents in the associated disease
conditions. On the one hand, antibodies to the NR1 subunit of
N-methyl-D-aspartate receptors have been suggested to cause internalization and
loss of these receptors without any cell destruction. This mechanism can explain
the reversible functional effects caused by these antibodies. On the other hand,
antibody- and complement-mediated destructive, and the irreversible effects of
antibodies against the voltage-gated potassium channel antigens have been noted.
These emerging findings make it plausible that immunological therapies,
preferably early after characterization of the antibodies, offer opportunities to
restore the health of affected patients.
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